Bottom Line:
EMPD in some cases has an associated adenocarcinoma, which has been associated with a worse prognosis and high mortality.Our surgical approach is considered in literature as the best one for those cases, followed by the oncological treatment.Those patients need to be better studied and more attention should be paid to the clinical presentation.

Affiliation: General Surgery Unit, Department of Emergency and Organ Transplantation-University of Bari, Italy.

ABSTRACT

Introduction: Extramammary Paget's disease (EMPD) occurs commonly in perineum, vulva and perineal region and is considered as a complex disorder due to different clinical and histological features.

Presentation of case: A 61 years old woman had a dermatologic evaluation for anal itch and underwent a skin biopsy with diagnostic of Paget disease in perianal region. Pelvic magnetic resonance showed a huge tumor which involved skin, derma and gluteus and she firstly refused any surgical treatment. One year later, because of increasing of the tumor and bleeding, she underwent the surgical procedure with a complete excision, resection of the skin of the anus, inguinal lymphadenectomy and left colostomy. Because of lymph nodes metastasis, a VLS Miles was sequentially performed. She started oncological follow up that showed liver, lung and pelvic metastasis. She survived for 24 months after surgery.

Discussion: In literature, less than 200 cases of perineal Paget's disease have been reported. EMPD in some cases has an associated adenocarcinoma, which has been associated with a worse prognosis and high mortality. Wrong diagnosis and a superficial consideration of a benign evolution should be considered as the first mistake in clinical practice.

Conclusion: Our surgical approach is considered in literature as the best one for those cases, followed by the oncological treatment. Those patients need to be better studied and more attention should be paid to the clinical presentation.

Mentions:
After a dermatologic evaluation for anal itch, a 61 years old woman underwent a skin biopsy whose subsequent histological exam deposed for Paget disease in perianal region Fig 1. After that biopsy, the patient has been controlled for several months by the plastic surgeons without any improvement, and one year later, she performed a colonoscopy with evidence of left colonic diverticulitis and a perianal mass. Primarily she refused any surgical treatment proposed. During the same time she underwent a pelvic Magnetic Resonance which described inhomogeneous tissue with high enhancement and a depth of 3.8 cm and a length of 8 cm on the right side of the anus which involved skin, derma and gluteus. During the following year, because of the bleeding and the increase of the mass, she decided to be submitted to surgery. A complete excision of the tumor was performed associated to the resection of the skin of the anus, inguinal lymphadenectomy and left colostomy. The subsequent specimen was positive for lymph node metastasis, with infiltration of the internal sphincter and the histological pattern was a mildly differentiated adenocarcinoma with focal aspect of Paget. The resection margins were positive for tumor. She performed a TB CT scan that didn't show any evidence of metastasis. After this histological result she was readmitted to the operating room to perform a VLS Miles resection end excision of the big lip of the vagina (Fig. 2) with a terminal left colostomy. The definitive histological exam was positive for metastasis in 3 perirectal lymph nodes and massive infiltration of the right big lip of the vagina. The patient was dismissed after 20 days. She started oncologic follow up with laboratory data and total body CT scan that showed liver and abdominal metastasis. A PET-CT scan showed different pathological backlog of the radiopharmaceutical at the liver (SUV max 6,4), and at the inguinal region (SUV max 3,4), ascribable to adenopathy. During the following 12 months, she was submitted to 6 cycles of Chemotherapy (CHT) according to the XELOX protocol and also she underwent an UltraSound scan of the right inferior leg that highlighted a total thrombosis of the right external iliac vein and of the femoral vein and for this reason she started therapy with low molecular weight heparin. After the first CHT step she started other 3 cycles with FOLFOX4-Bevacizumab. Unfortunately laboratory data showed a constant increase of CA 19,9, TPA and liver function tests. Last follow up was made at 24months after the surgery and the CT scan showed an increase of liver, lung and perianal metastasis. After one month the patient died because of hepatic failure.

Mentions:
After a dermatologic evaluation for anal itch, a 61 years old woman underwent a skin biopsy whose subsequent histological exam deposed for Paget disease in perianal region Fig 1. After that biopsy, the patient has been controlled for several months by the plastic surgeons without any improvement, and one year later, she performed a colonoscopy with evidence of left colonic diverticulitis and a perianal mass. Primarily she refused any surgical treatment proposed. During the same time she underwent a pelvic Magnetic Resonance which described inhomogeneous tissue with high enhancement and a depth of 3.8 cm and a length of 8 cm on the right side of the anus which involved skin, derma and gluteus. During the following year, because of the bleeding and the increase of the mass, she decided to be submitted to surgery. A complete excision of the tumor was performed associated to the resection of the skin of the anus, inguinal lymphadenectomy and left colostomy. The subsequent specimen was positive for lymph node metastasis, with infiltration of the internal sphincter and the histological pattern was a mildly differentiated adenocarcinoma with focal aspect of Paget. The resection margins were positive for tumor. She performed a TB CT scan that didn't show any evidence of metastasis. After this histological result she was readmitted to the operating room to perform a VLS Miles resection end excision of the big lip of the vagina (Fig. 2) with a terminal left colostomy. The definitive histological exam was positive for metastasis in 3 perirectal lymph nodes and massive infiltration of the right big lip of the vagina. The patient was dismissed after 20 days. She started oncologic follow up with laboratory data and total body CT scan that showed liver and abdominal metastasis. A PET-CT scan showed different pathological backlog of the radiopharmaceutical at the liver (SUV max 6,4), and at the inguinal region (SUV max 3,4), ascribable to adenopathy. During the following 12 months, she was submitted to 6 cycles of Chemotherapy (CHT) according to the XELOX protocol and also she underwent an UltraSound scan of the right inferior leg that highlighted a total thrombosis of the right external iliac vein and of the femoral vein and for this reason she started therapy with low molecular weight heparin. After the first CHT step she started other 3 cycles with FOLFOX4-Bevacizumab. Unfortunately laboratory data showed a constant increase of CA 19,9, TPA and liver function tests. Last follow up was made at 24months after the surgery and the CT scan showed an increase of liver, lung and perianal metastasis. After one month the patient died because of hepatic failure.

Bottom Line:
EMPD in some cases has an associated adenocarcinoma, which has been associated with a worse prognosis and high mortality.Our surgical approach is considered in literature as the best one for those cases, followed by the oncological treatment.Those patients need to be better studied and more attention should be paid to the clinical presentation.

Affiliation:
General Surgery Unit, Department of Emergency and Organ Transplantation-University of Bari, Italy.

ABSTRACT

Introduction: Extramammary Paget's disease (EMPD) occurs commonly in perineum, vulva and perineal region and is considered as a complex disorder due to different clinical and histological features.

Presentation of case: A 61 years old woman had a dermatologic evaluation for anal itch and underwent a skin biopsy with diagnostic of Paget disease in perianal region. Pelvic magnetic resonance showed a huge tumor which involved skin, derma and gluteus and she firstly refused any surgical treatment. One year later, because of increasing of the tumor and bleeding, she underwent the surgical procedure with a complete excision, resection of the skin of the anus, inguinal lymphadenectomy and left colostomy. Because of lymph nodes metastasis, a VLS Miles was sequentially performed. She started oncological follow up that showed liver, lung and pelvic metastasis. She survived for 24 months after surgery.

Discussion: In literature, less than 200 cases of perineal Paget's disease have been reported. EMPD in some cases has an associated adenocarcinoma, which has been associated with a worse prognosis and high mortality. Wrong diagnosis and a superficial consideration of a benign evolution should be considered as the first mistake in clinical practice.

Conclusion: Our surgical approach is considered in literature as the best one for those cases, followed by the oncological treatment. Those patients need to be better studied and more attention should be paid to the clinical presentation.